Multifocal intradural extramedullary ependymoma. Case report

In this paper, the authors present the case of a patient with multifocal intradural extramedullary ependymoma, and they review 18 previously reported cases. A 32-year-old man presented to the authors' institution with a 1-month history of partial medullary syndrome. Magnetic resonance imaging of the neuraxis revealed multifocal intradural extramedullary lesions at the bulbomedullary junction and C2-3, T5-11, L-2, L-4, L-5, and sacrum. Histological examination revealed a WHO Grade II ependymoma. The literature survey yielded 18 cases of ependymoma at the same location; none of them were multifocal at presentation. The authors analyzed the epidemiological, clinical, and surgical features of all 19 cases reported to date, including the present case. Patients' ages ranged from 24 to 69 years; 15 patients were women and 4 were men. The time elapsed from symptom onset to diagnosis ranged from 1 month to 8 years. Pain (in 13 patients) and medullary syndrome (in 12) were reported as the initial symptoms (information was not provided for 1 patient). Tumors were predominantly located in the thoracic spine (11), but they also occurred in the cervicothoracic (3), cervical (2), and lumbar (2) spine. The remaining tumor was multifocal. Solitary extramedullary tumors were found intraoperatively in 13 patients; 3 were described as exophytic and 3 as extramedullary with some degree of medullary invasion. Histological examination revealed 9 WHO Grade II tumors, 4 Grade III tumors, and 1 myxopapillary tumor. Data obtained for the remaining cases proved inconclusive. The clinical condition improved in 11 patients, remained stable in 2, and worsened (recurrence or progression) in 6. Of the 4 patients with Grade II tumors who presented with recurrence or neuraxis spreading, 3 had meningeal infiltration or adhesion to the pia mater, which does not rule out the possibility of neoplastic remnants in that area. Intradural extramedullary ependymomas are rare, they predominate in women in the 5th decade of life, and pain is the most frequent initial symptom. The extent of resection and the presence of meningeal infiltration seem to be key determinants of prognosis. The present case is the first intradural extramedullary ependymoma (with the exception of those occurring at the conus medullaris and terminal filum) with multiple lesions at presentation.     

Intradural extramedullary tuberculoma of the cervical spine. Case report

A case is reported of a 45-year-old man who developed quadriplegia following a trivial motor-vehicle accident. Investigation including computerized tomography (CT) of the cervical spine revealed a large calcified lesion displacing the spinal cord and nerve roots, which proved to be a tuberculoma. The case is unusual in regard to the age of the patient, the size, location, and nature of the lesion, the mode of presentation, and the delineation of the lesion by CT scanning.     

Intradural extramedullar tuberculoma of spinal cord. Case report

A case of intradural tuberculoma in an infant and the patient was diagnosed operated on successfully. The author's advice is to perform surgery followed by antituberculosis treatment during the recovery period.     

Type A intradural spinal arteriovenous fistula. Case report

In this report, the authors present the case of a patient with a unique type of spinal arteriovenous fistula. Both the location and venous angioarchitecture of this variant are uncommon, making diagnosis of the lesion challenging and raising particular management issues. The authors discuss this unusual lesion and describe its imaging features and surgical findings, as well as highlight its pathological abnormalities.     

Pituitary carcinoma recurrent to the lumbar intradural extramedullary space: case report

Context

Pituitary tumors are rare, and pituitary carcinomas are rarer still. Prognosis is poor, with less than 50% of patients surviving past 1 year after diagnosis. In this case of spinal metastasis from an adrenocorticotropic hormone-secreting pituitary carcinoma, the intradural extramedullary metastases recurred in the same lumbar area 6 years apart.

Findings

Fourteen years prior to presentation in our clinic, a 48-year-old woman was diagnosed with pituitary adenoma which was treated with resection followed by radiation. Eight years later, an intradural extramedullary spinal drop metastasis at L2–L3 was again treated with resection and radiation. Three years later, magnetic resonance imaging (MRI) revealed a mass encasing the right carotid artery, which was treated for 1 year with chemotherapy using temozolomide (Temodar). Three years later, MRI showed intradural extramedullary metastases at the L3–L4 intervertebral disc space and behind the L3 vertebral body; treatment was again resection followed by radiation. Back pain and weakness resolved after surgery and her neurological examination returned to baseline. There was no evidence of recurrence 1 year after surgery.

Conclusion/clinical relevance

In this unusual case, this pituitary carcinoma metastasized twice in 6 years to virtually the same intradural extramedullary lumbar region. Surgical resection of these masses aided in relieving neurological symptoms and prolonging life.     

胸椎管内髓外硬膜下原发性黑色素细胞瘤1例报告

脊膜黑色素瘤是一种少见的含色素的中枢神经系统肿瘤,来源于软脊膜的黑色素细胞,可发生于脑脊膜任何部位[1].我科收治胸椎管内髓外硬膜下原发性黑色素细胞瘤1例,报告如下. 患者男性,43岁.因腰背部束带感、双下肢乏力3个月,双下肢麻木2个月于2011年10月7日入院.患者于3个月前无明显诱因出现腰背部束带感,双下肢乏力,在当地医院就诊,诊断不明,未给予特殊处理.2个月前上述情况加重,并出现双下肢麻木,行走不稳,有踏空感,且上述情况逐渐加重,遂来我院就诊.     

Concurrent intracranial and spinal cord arteriovenous malformations. Case report.

The authors report the case of a patient with subarachnoid hemorrhage in whom an intracranial dural arteriovenous malformation coexisted with a spinal arteriovenous malformation. The latter was considered to be the source of the hemorrhage by clinical and radiographic criteria. It is concluded that patients with subarachnoid hemorrhage who show no suitable intracranial source for their bleed in some instances should be investigated for a spinal origin of hemorrhage.     

Spinal cord compression due to intradural extramedullary aspergilloma and cyst: a case report

A case of thoracic spinal cord compression caused by intradural extramedullary cyst associated with aspergilloma is presented. The specific diagnosis was extremely difficult and required specialized tissue studies. Clinical suspicion of fungal infection, surgical intervention for spinal cord decompression, and tissue biopsy and relentless diagnostic laboratory studies are particular features of this case.     

Metastatic intradural extramedullary spinal cord tumor from ovarian cancer: A case report with a literature review

Context: Metastatic intradural extramedullary spinal cord tumors are extremely rare.Findings: A 76-year-old woman presented with intractable neck pain. Three years earlier, she had been treated for ovarian cancer with bilateral salpingo-oophorectomy. A year later, she underwent resection of a brain metastasis. Magnetic resonance imaging (MRI) showed an encapsulated intradural extramedullary mass at C4–C5. C4–C5 hemilaminectomy, tumor resection, and biopsy were performed. Histological examination of the resection revealed an adenocarcinoma. After surgery, her intolerable neck-shoulder pain was fully resolved, and she had no difficulties with daily living activities. However, two months later, she underwent gamma knife radiosurgery for the recurrent metastatic brain tumor, and four months later, she died from cachexia.Conclusion: Although cases of metastatic intradural extramedullary spinal tumors from ovarian cancer are extremely rare, their possibility should be considered in the differential diagnosis. A history of brain metastases and enhancement on T1-weighted MRI were helpful for making an accurate diagnosis.     

Primary intradural extramedullary ependymoma: case report and review of the literature

STUDY DESIGN: The authors report the ninth case in the literature of a primary intradural extramedullary ependymoma of the spinal cord. OBJECTIVE: To discuss surgical treatment and the physiopathologic hypothesis of this localization on the basis of the results of the present study and a review of the literature. SUMMARY OF BACKGROUND DATA: Ependymoma is a glial tumor known to arise in the central nervous system. Intradural extramedullary location of this neoplasm has been exceptionally described previously. METHODS: A 43-year-old woman was admitted to the authors' institution with an history of progressive paraplegia. Neurologic examination showed sensory loss below T1 and bladder disturbances. Magnetic resonance imaging revealed an enhanced thoracic intradural extramedullary tumor, extending from T1-T8. No other lesion in the central nervous system was found. Emergency surgical resection was performed. RESULTS: Surgery gave confirmation of an encapsulated extramedullary tumor without attachment to the spinal cord or to the dura mater. Total removal was achieved under microscope. The postoperative course was uneventful, with complete neurologic recovery 3 months later. The patient has been well for 24 months of follow-up evaluation, without evidence of recurrence on magnetic resonance images. Histologic examination revealed the tumor as a benign ependymoma. CONCLUSION: The encapsulated feature, the lack of attachment to the central nervous system, and the absence of other neoplastic processes within the brain or the spinal cord suggested that this lesion is a primary tumor developed from ectopic ependymal cells.     

Spinal intradural extramedullary cavernous angioma: case report and review of the literature

STUDY DESIGN: Case report and review of the literature. OBJECTIVE: To report an unusual case of a spinal intradural extramedullary cavernous angioma (CA), located at the cauda equina, and to compare it with the previously published 22 surgically treated cases in the literature. SETTING: Ankara, Turkey. METHOD: A 67-year-old man presented with a 4-month history of back pain and sciatica and a 4-week history of progressive lower extremity paresthesia, difficulty walking, and loss of bladder and bowel sphincter control. Neurological examination revealed bilateral hypoesthesia below the T12 dermatome with spastic paraparesis. Magnetic resonance imaging (MRI) of the cauda equina revealed a heterogeneous enhancing intradural extramedullary mass obliterating the spinal canal and expanding the spinal cord. T12 laminectomy and total tumor removal were performed without additional neurological deficit. Pathological examination confirmed the diagnosis of a CA. RESULT: The patient's neurological status improved during postoperative recovery. He was ambulatory without assistance and regained full sphincter control on the eighth postoperative week. CONCLUSION: CAs of the spinal cord are extremely rare lesions. Typically, they present with low back pain and sciatica, neurological deficits, or as a subarachnoid hemorrhage. These lesions have characteristic features on MRI and should be considered in the differential diagnosis of intradural spinal lesions. Following a thorough literature review of reported cases, the authors propose that for patients presenting with severe preoperative neurological signs, immediate microsurgical tumor excision or decompression increases the chance of neurological improvement.     

Presentation of cauda equina syndrome due to an intradural extramedullary abscess: a case report

Background contextCauda equina syndrome is caused by compression or injury to the nerve roots distal to the level of the spinal cord. This syndrome presents as low back pain, motor and sensory deficits in the lower extremities, and bladder as well as bowel dysfunction. Although various etiologies of cauda equina syndrome have been reported, a less common cause is infection.PurposeTo report a case of cauda equina syndrome caused by infection of an intradural extramedullary abscess with Staphylococcus aureus.Study design/settingCase report and review of the literature.MethodsThe literature regarding the infectious causes of cauda equina syndrome was reviewed and a case of cauda equina syndrome caused by infection of an intradural extramedullary abscess with Staphylococcus aureus was reported.ResultsA 37-year-old woman, with history of intravenous drug abuse, hepatitis C, and hepatitis B, presented with low back pain lasting 2 months, lower extremity pain, left greater than right with increasing weakness and difficulty ambulating, and urinary and fecal incontinence. Her presentation was consistent with cauda equina syndrome. The patient underwent a T12–L2 laminectomy, and intradural exploration revealed an abscess. Methicillin-resistant Staphylococcus aureus was found on wound culture.ConclusionsCauda equina syndrome, presenting as a result of spinal infection, such as the case reported here, is extremely rare but clinically important. Surgical intervention is generally the recommended therapeutic modality.     

Paraplegia due to extramedullary hematopoiesis in thalassemia. Case report

Surgical excision followed by radiotherapy has been the recommended treatment for paraplegia due to extramedullary hematopoiesis in patients with beta-thalassemia. The authors report the successful treatment of such a case by partial excision and repeated blood transfusions.     

Intramedullary and extramedullary schwannoma of the cervical spinal cord—Case report

Intramedullary schwannomas and neurofibromas are rare tumors. Only two cases have been reported as having both an intramedullary and extramedullary component.

We have managed the case of a 15-year-old girl with a schwannoma that appeared to track along the sensory nerve root into the spinal cord. The clinical presentation in this case was that of motor weakness and atrophy, sensory abnormalities, and, late in the course, pain.

Magnetic resonance imaging with gadolinium enhancement was better than myelography and computed tomography at delineating the intramedullary extent of the tumor. The tumor was removed microsurgically at two operative sittings. Reports of this unusual pathology are reviewed.     

An extramedullary plasmacytoma originating from the thoracic spinal cord: magnetic resonance imaging findings. Case report

Extramedullary plasmacytoma (EMP) is a rare tumor. The authors report a case of an EMP originating in the thoracic spinal cord. To their knowledge this is the first report in which an EMP originating in this location is described. Isointense and hyperintense signals were seen on T1-weighted and T2-weighted magnetic resonance images, respectively, with marked irregular contrast enhancement and extensive edema. No evidence of bone marrow involvement or multiple myeloma was noted. The histological findings were compatible with an EMP.     

Regression of intracranial rosai-dorfman disease following corticosteroid therapy. Case report

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder usually presenting with massive, painless lymphadenopathy. Extranodal involvement has been reported including at least 50 cases affecting the central nervous system (CNS). The treatment of CNS RDD as reported in the literature has primarily involved a surgical technique. The authors report on the case of a 53-year-old man presenting with multiple skull base lesions mimicking meningiomas. The patient suffered visual deterioration and underwent a right orbitopterional craniotomy as well as optic nerve decompression. Histopathological analysis revealed histiocytic cells and emperipolesis consistent with RDD. Following surgery, corticosteroid agents were administered, leading to marked resolution of both the remaining surgically untreated lesions and the balance of the patient's symptoms. This report represents the first case of the resolution of intracranial RDD following corticosteroid therapy. Corticosteroid agents should be considered an effective option in the treatment of CNS RDD.